Myeloid Lymphoid Bone Marrow Disorders

Question 1

Increase in number of circulating neutrophils

Answer 1

Neutrophilia

Question 2

Cytoplasmic findings of neutrophils associated with bacteria infections (3)

Answer 2

1. Toxic granulation
2. Dohle bodies: rough ER
3. Cytoplasmic vacuolation

Question 3

Extreme leukocytosis resulting in high WBC that simulates leukemia but is due to infection or another underlying disorder.

May resemble chronic myelogenous leukemia

Answer 3

Leukemoid reaction

Question 4

Malignant clonal neoplasm of multipotent myeloid stem cells characterized by accumulation of myeloblasts in bone marrow and blood

Answer 4

Acute Myeloid Leukemia

Question 5

Block in differentiation of leukemic stem cells

Answer 5

Acute myeloid leukemia

Question 6

t (15;17) seen in ____

Treatment by ___

Answer 6

Acute promyelocytic leukemia

Treatment by all-trans-retinoic acid (ATRA)

Question 7

Clinical features of acute myeloid leukemia

Answer 7

Fatigue: anemia
Infection: granulocytopenia
Bleeding: thrombocytopenia

Question 8

Disseminated intravascular coagulation (DIC) common in ___

Answer 8

acute promyelocytic leukemia with t(15;17)

Question 9

Infiltration of gums seen in ____

Answer 9

acute myelomonocytic leukemia

Question 10

Prognosis of AML
Initial:
Long term survival:

Answer 10

60-80% reach initial remission w/ combination chemotherapy BUT relapses common
Long term survival: 1/3 pt age 18-60.
Pt w/ t(15;17) have 80% survival.
Unfavorable cytogenetics have

Question 11

Positive cytochemical stains for myeloperoxidase

Answer 11

Acute Myeloid Leukemia

Question 12

Negative cytochemical stain for myeloperoxidase

Answer 12

Acute Lymphoblastic Leukemia

Question 13

Most (80%) acute lymphoblastic leukemia have precursor B cells that express:

Answer 13

CD19, CD10, TdT

Question 14

Less common acute lymphoblastic leukemia have precursor T cells that express:

Answer 14

CD7, CD2, TdT

Question 15

Prognosis for B-ALL w/ hyperdiploidy

Answer 15

Favorable

Question 16

Prognosis of B-ALL with t(12;21) TEL-AML1

Answer 16

Favorable

Question 17

Prognosis of B-ALL with t(9;22) BCR-ABL1, 11q23 rearrangements

Answer 17

Unfavorable

Question 18

Prognosis of B-ALL with hypodiploidy

Answer 18

Unfavorable

Question 19

Most common childhood tumor

Answer 19

Acute lymphoblastic leukemia

Question 20

Clinical presentation of ____:
Fatigue, infections, bleeding
Bone pain, lymphadenopathy, hepatosplenomegaly, CNS symptoms

Answer 20

Acute lymphoblastic leukemia

Question 21

Chronic myeloproliferative neoplasm characterized by predominately granulocytic differentiation. Increased neutrophils granulocytic precursors (some), basophila, eosinophilia.

Answer 21

Chronic Myelogenous Leukemia

Question 22

15% of leukemia in adults, rare in children. Onset of 55 y/o.

Answer 22

Chronic Myelogenous Leukemia

Question 23

Often asymptomatic at diagnosis. Lethargy, fatigue, weight loss. May have splenomegaly.

Answer 23

Chronic Myelogenous Leukemia

Question 24

Left shift with full range of myeloid precursors circulating in peripheral blood. Basophilia. Eosinophilia.

Answer 24

Chronic Myelogenous Leukemia

Question 25

Morphology of bone marrow in CML

Answer 25

Hypercellular bone marrow. Increased number of maturing myeloid cells. Normal myeloid cell morphology. NO increase in blast cells. Barely any fat.

Question 26

marked neutropenia, usually

Answer 26

Agranulocytosis | Predisposition to infection

Question 27

Has Philadelphia chromosome from t(9;22)(q34q11) resulting in long arm chromosome 9 and short chromosome 22

Answer 27

CML

Question 28

Genes that are fused in CML | Leads to:

Answer 28

BCR/ABL | Production of abnormal tyrosine kinase with altered activity

Question 29

Clinical manifestation: 50% are asymptomatic Lethargy, fatigue, weight loss Splenomegaly (80%)

Answer 29

Chronic Myelogenous Leukemia

Question 30

Treatment of CML

Answer 30

``` Imatinib mesylate (Gleevec): blocks tyrosine kinase Continue indefinitely 90% pt go into remission ```

Question 31

Blast phase of CML = Symptoms: Lineage: Treatment:

Answer 31

>20% blasts in peripheral blood or marrow Fever, night sweats, bone pain, hemorrhage, anemia 80%: myeloid lineage 20%: lymphoid lineage Similar to AML: chemotherapy

Question 32

Clonal lymphoproliferative disorder of morphologically "mature" lymphocytes If only involves lymph nodes =

Answer 32

Chronic Lymphocytic Leukemia Small Lymphocytic Lymphoma

Question 33

Morphology of Chronic Lymphocytic Leukemia: | Characteristic feature of CLL:

Answer 33

Small, condensed nuclear chromatin, monomorphic cells | B cells express CD5 (T cell marker)

Question 34

CLL seen usually in:

Answer 34

Pt greater than 60 y/o | More common in males

Question 35

Clinical features of chronic lymphocytic leukemia

Answer 35

Susceptibility to infection Anemia Thrombocytopenia

Question 36

Dyspoiesis =

Answer 36

abnormal morphologic features in maturing myeloid, erythroid and megakaryocytic cells

Question 37

Clonal myeloid stem cell disorders characterized by peripheral blood cytopenias, ineffective hematopoiesis and dyspoiesis

Answer 37

Myelodysplastic Syndrome (MDS)

Question 38

Trisomies and deletions are common in ___

Answer 38

Myelodysplastic Syndromes

Question 39

Morphology of Myelodysplastic Syndromes

Answer 39

Pancytopenia w/ dyspoietic features of RBC, granulocytes and platelets Dyspoietic features of granulocytic precursors, erythroid precursors, megakaryocytes Increased myeloblasts BUT always less than 20% Hypercellular bone marrow

Question 40

Chronic Myeloproliferative Neoplasms caused by:

Answer 40

mutation in JAK2 gene (Jak2 V617F) --> constituativelly on pathway even without ligands --> no proliferative control of stem cells See in all polycythemia Half in essential thrombocythemia and primary myelofibrosis

Question 41

Clonal proliferation of multipotent myeloid stem cells that result in increased number of differentiated granulocytes, red blood cells or platelets.

Answer 41

Chronic Myeloproliferative Neoplasms

Question 42

Types of Chronic Myeloproliferative Neoplasms

Answer 42

Chronic myelogenous leukemia (CML) Polycythemia vera Essential thrombocythemia Primary myelofibrosis

Question 43

Chronic myeloproliferative neoplasm associated w/ thrombocytosis and increased megakaryocytes in bone marrow.

Answer 43

Essential thrombocythemia

Question 44

Least likely to transform to AML or myelofibrosis

Answer 44

Essential Thrombocythemia

Question 45

Clinical features of essential thrombocythemia: | Typically seen in:

Answer 45

Thrombosis or hemorrhage Splenomegaly Adults (50-60 y/o)

Question 46

Morphology of essential thrombocythemia

Answer 46

Increase platelet count Variation in size of platelets increased large atypical megakaryocytes in bone marrow

Question 47

Characteristic feature of primary myelofibrosis

Answer 47

Tear drop RBC

Question 48

A clonal myeloproliverative neoplasm characterized by proliferation of predominantly megakaryocytes and granulocytes in the bone marrow. Reactive deposition of fibrous connective tissue with extramedullary hematopoiesis.

Answer 48

Primary myelofibrosis

Question 49

Extramedullary hematopoiesis in:

Answer 49

primary myelofibrosis

Question 50

Morphology of primary myelofibrosis:

Answer 50

Marked splenomegaly: extramedullary hematopoiesis Tear drop RBC Leukoerythroblastic reaction: immature myeloid cells and nucleated RBC in blood

Question 51

Morphology of bone marrow in primary myelofibrosis

Answer 51

Cellular stage: trilineage hyperplasia --> hypercelluar marrow Increased neutrophils and clusters of large atypical megakaryocytes Fibrotic stage: Hypocellular marrow

Question 52

Clinical features of primary myelofibrosis: Seen in: Survival:

Answer 52

Splenomegaly Insidious onset Middle age and elderly Survival 3 years: 5-20% progress to acute leukemia

Question 53

Chronic myeloproliferative neoplasm characterized by increased red cells in peripheral blood and bone marrow. Possible leukocytosis and thrombocytosis.

Answer 53

Polycythemia vera

Question 54

Diseases characterized by clonal proliferation of terminally differentiated B cells capable of secreting immunoglobulin or portions of immunoglobulin (M-protein).

Answer 54

Plasma cell neoplasms

Question 55

Bone marrow based plasma cell neoplasm associated with an M-protein in serum and/or urine.

Answer 55

Plasma cell myeloma (multiple myeloma)

Question 56

Symptoms of symptomatic myeloma

Answer 56

``` End organ damage "CRAB": hyperCalcemia Renal insufficiency Aneima Bone or lytic lesions ```

Question 57

Clinical features of plasma cell myeloma: Seen in: Survival:

Answer 57

Renal damage Recurrent infections Bone pain and fractures due to lytic lesions 50+ y/o Incurable: 5 year survival

Question 58

Pathological features of plasma cell myeloma:

Answer 58

Blood smear: rouleaux formation | Bone marrow: increased number of clonal plasma cells